Baseline characteristics and evolution of Brazilian patients with atypical hemolytic uremic syndrome: first report of the Brazilian aHUS Registry.

Background: Atypical hemolytic uremic syndrome (aHUS) is an ultra-rare disease. Therefore, studies involving large samples are scarce, making registries powerful tools to evaluate cases. We present herein the first analysis of the Brazilian aHUS Registry (BRaHUS). Methods: Analysis of clinical, laboratory, genetic and treatment data from patients inserted in the BRaHUS, from 2017 to 2020, as an initiative of the Rare Diseases Committee of the Brazilian Society of Nephrology. Results: The cohort consisted of 75 patients (40 adults and 35 pediatric). There was a predominance of women (56%), median age at diagnosis of 20.7 years and a positive family history in 8% of cases. Renal involvement was observed in all cases and 37% had low C3 levels. In the <2 years of age group, males were predominant. Children presented lower levels of hemoglobin (P = .01) and platelets (P = .003), and higher levels of lactate dehydrogenase (LDH) (P = .004) than adults. Genetic analysis performed in 44% of patients revealed pathogenic variants in 66.6% of them, mainly in CFH and the CFHR1-3 deletion. Plasmapheresis was performed more often in adults (P = .005) and 97.3% of patients were treated with eculizumab and its earlier administration was associated with dialysis-free after 3 months (P = .08). Conclusions: The cohort of BRaHUS was predominantly composed of female young adults, with renal involvement in all cases. Pediatric patients had lower hemoglobin and platelet levels and higher LDH levels than adults, and the most common genetic variants were identified in CFH and the CFHR1-3 deletion with no preference of age, a peculiar pattern of Brazilian patients.

Secondary household transmission of SARS-CoV-2 among children and adolescents: Clinical and epidemiological aspects.

OBJECTIVE: To evaluate the secondary attack rate (SAR) in children and adolescents, contacts of essential activities workers who were infected by SARS-CoV-2; and to describe associated clinical and epidemiological data. METHODS: A cross-sectional study conducted in children and adolescents aged 5 to 19 years of age, that were household contacts of parents and other relatives who were infected by SARS-CoV-2 in the city of Goiânia, Central Brazil, from March to October 2020. Sociodemographic and clinical data were collected from all participants. Nasopharyngeal and oropharyngeal swabs were collected and tested for SARS-CoV-2 RNA using real-time reverse transcription polymerase chain reaction (RT-PCR). Factors associated with SARS-CoV-2 infection and SAR were analyzed using Poisson regression. RESULTS: A total of 267 children and adolescents were investigated. The prevalence of SARS-CoV-2 RNA by the real-time RT-PCR test and/or the presence of COVID-19 associated symptoms (anosmia/ageusia and flu syndrome) was 25.1% (95.0% Confidence Interval [95.0% CI] = 20.3-30.6). More than half (55.1%) of the participants had sygns and symptoms. The most prevalent signs and symptoms in positive individuals were nasal congestion (62.7%), headache (55.2%), cough (50.8%), myalgia (47.8%), runny nose (47.8%), and anosmia (47.8%). The Poisson model showed that the following signs or symptoms were associated with SARS-CoV-2 infection: fever, nasal congestion, decreased appetite, nausea, anosmia, and ageusia. Families that had more than one infected adult, in addition to the index case, presented greater transmissibility to children and adolescents. CONCLUSIONS: Our results contribute to the hypothesis that children and adolescents are not important sources of transmission of SARS-CoV-2 in the home environment during a period of social distancing and school closure; even though they are susceptible to infection in the household (around » of our study population).

Inflammatory cytokines and lipid profile in children and adolescents with nephrotic syndrome receiving L. Plantarum: a randomized, controlled feasibility trial.

METHODS: This study aimed to evaluate the efficacy of the action of the Lactobacillus Plantarum probiotic as a immunomodulatory and hypolipidemic agent in dyslipidemic nephrotic children and adolescents. This is a randomized, double-blind, placebo-controlled clinical trial in pediatric, compensated or partially compensated nephrotic syndrome and dyslipidemic subjects undergoing regular outpatient follow-up. Serum lipid and TNF-α (proinflammatory) and IL-10 (anti-inflammatory) cytokine variations were evaluated. Cytokines were analyzed by enzyme-linked immunosorbent assay (ELISA). RESULTS: In the probiotic group there was a tendency to reduce TNF-α levels and increase IL-10 levels when compared to controls. Regarding the lipid profile, there was a decrease in serum triglyceride (6.0 mg / dL) and total cholesterol (41.5 mg / dL) levels in the probiotic group when compared to baseline levels, while in the control group there was an increase in serum triglyceride (49.5 mg / dL) and total cholesterol (8.0 mg / dL) levels, respectively. CONCLUSION: Preliminary results suggest that L. Plantarum showed an immunomodulatory and hypolipidemic effect in nephrotic and dyslipidemic pediatric subjects.

Inflammatory cytokines and lipid profile in children and adolescents with nephrotic syndrome receiving L. Plantarum: a randomized, controlled feasibility trial

SUMMARY This study aimed to evaluate the efficacy of the action of the Lactobacillus Plantarum probiotic as a immunomodulatory and hypolipidemic agent in dyslipidemic nephrotic children and adolescents. METHODS: This is a randomized, double-blind, placebo-controlled clinical trial in pediatric, compensated or partially compensated nephrotic syndrome and dyslipidemic subjects undergoing regular outpatient follow-up. Serum lipid and TNF-α (proinflammatory) and IL-10 (anti-inflammatory) cytokine variations were evaluated. Cytokines were analyzed by enzyme-linked immunosorbent assay (ELISA). RESULTS: In the probiotic group there was a tendency to reduce TNF-α levels and increase IL-10 levels when compared to controls. Regarding the lipid profile, there was a decrease in serum triglyceride (6.0 mg / dL) and total cholesterol (41.5 mg / dL) levels in the probiotic group when compared to baseline levels, while in the control group there was an increase in serum triglyceride (49.5 mg / dL) and total cholesterol (8.0 mg / dL) levels, respectively. CONCLUSION: Preliminary results suggest that L. Plantarum showed an immunomodulatory and hypolipidemic effect in nephrotic and dyslipidemic pediatric subjects.

RESUMO: O objetivo deste estudo foi avaliar a eficácia da ação do probiótico Lactobacillus Plantarum como um agente imunomodulador e hipolipemiante em crianças e adolescentes dislipidêmicos com síndrome nefrótica. MÉTODOS: Este é um estudo randomizado, duplo-cego, controlado com placebo e realizado em pacientes pediátricos com síndrome nefrótica, compensados ou parcialmente compensados e dislipidêmicos passando por acompanhamento ambulatorial. Variações no lipídio sérico e nas citocinas TNF-α (pró-inflamatória) e IL-10 (anti-inflamatória) foram avaliadas. As citocinas foram analisadas por ensaio imunoenzimático (ELISA). RESULTADOS: No grupo probiótico, houve uma tendência de redução dos níveis de TNF-α e de aumento dos níveis de IL-10, quando comparado ao controle. Em relação ao perfil lipídico, houve uma diminuição nos níveis séricos de triglicérides (6,0 mg/dL) e colesterol total (41,5 mg/dL) no grupo probiótico em comparação aos níveis basais, enquanto no grupo de controle houve um aumento nos níveis séricos de triglicérides (49,5 mg/dL) e colesterol total (8,0 mg/dL). CONCLUSÃO: Os resultados preliminares sugerem que o L. Plantarum tem um efeito imunomodulador e hipolipemiante em pacientes pediátricos dislipidêmicos e com síndrome nefrótica.

A Methodological Integrated Approach to Analyse Climate Change Effects in Agri-Food Sector: The TIMES Water-Energy-Food Module.

The European Union's 2030 climate and energy policy and the 2030 Agenda for Sustainable Development underline the commitment to mitigate climate change and reduce its impacts by supporting sustainable use of resources. This commitment has become stricter in light of the ambitious climate neutrality target set by the European Green Deal for 2050. Water, Energy and Food are the key variables of the "Nexus Thinking" which face the sustainability challenge with a multi-sectoral approach. The aim of the paper is to show the methodological path toward the implementation of an integrated modeling platform based on the Nexus approach and consolidated energy system analysis methods to represent the agri-food system in a circular economy perspective (from the use of water, energy, biomass, and land to food production). The final aim is to support decision-making connected to climate change mitigation. The IEA-The Integrated MARKAL-EFOM System (TIMES) model generator was used to build up the Basilicata Water, Energy and Food model (TIMES-WEF model), which allows users a comprehensive evaluation of the impacts of climate change on the Basilicata agri-food system in terms of land use, yields and water availability and a critical comparison of these indicators in different scenarios. The paper focuses on the construction of the model's Reference Energy and Material System of the TIMES model, which integrates water and agricultural commodities into the energy framework, and on the results obtained through the calibration of the model ß version to statistical data on agricultural activities.

The use of probiotics for improving lipid profiles in dyslipidemic individuals: an overview protocol.

BACKGROUND: Dyslipidemia is a major risk factor in triggering cardiovascular events, which can lead to the death of millions of people around the world. Thus, several pharmacological and non-pharmacological therapeutic strategies have been developed in recent decades with the objective of improving lipid profiles, including the use of probiotics. Therefore, the purpose of this protocol is to describe the steps that will guide the construction of an overview to demonstrate the scientific evidence of the efficacy of probiotics in improving the lipid profile of dyslipidemic individuals and to propose specific recommendations regarding their use. METHODS: The search will be conducted in the following databases: MEDLINE/PubMed, EMBASE, PROSPERO, Cochrane Library, CINAHL, JBI Database of Systematic Reviews and Implementation Reports, Google Scholar, and CADTH. Reviewers will select systematic evaluations and data analyses from randomized clinical trials that evaluated the effects of probiotics on lipid profiles. The studies will be analyzed for methodology quality by the AMSTAR 2 tool and risk of bias by ROBIS. The data will be extracted by three independent reviewers based on a data extraction sheet, which will include the most relevant variables for the analysis and interpretation of the results. The variables will be categorized and described in narrative form or in tables. DISCUSSION: There are some systematic reviews about the use of probiotics to prevent and/or treat dyslipidemia; however, their outcomes related to the ability of probiotics to improve lipid profiles are conflicting. So, an overview on this topic is needed to clarify this important issue. SYSTEMATIC REVIEW REGISTRATION: PROSPERO CRD42017080328.

[Evaluation of risk and protection factors associated with high blood pressure in children]. / Avaliação dos fatores de risco e proteção associados à elevação da pressão arterial em crianças.

BACKGROUND: Epidemiological studies have shown an increased prevalence of high blood pressure in pediatric patients. Today we know that risk factors can be detected during childhood and may help in preventing the disease. OBJECTIVE: To evaluate risk and protection factors related to high blood pressure in childhood. METHODS: We evaluated children aged 3 to 10 years, residing in the east and southwest sanitary districts of the City of Goiânia, Goiás, and obtained the following data: birth weight, breastfeeding, family history of high blood pressure and obesity, weight, height, body mass index (BMI), and blood pressure. We applied the Mann-Whitney U-test to these variables in order to compare pressure variation. RESULTS: In this sample, 519 children were evaluated, and 246 (47.4%) of them were male. The BMI assessment identified 109 (21%) overweight children, of which 53 (10.3%) were obese. Predominant and/or exclusive breastfeeding for less than 6 months was found in 242 (51.2%). The mean systolic pressure was significantly higher in children with exclusive and/or predominant breastfeeding for less than 6 months (p = 0.04), and in children with family history of high blood pressure (p = 0.05), and in overweight children (p <0.0001). These data were confirmed in multivariate analysis. CONCLUSION: In this sample, we observed that overweight and hereditary factors may be associated with elevated blood pressure, and that breastfeeding for more than 6 months seems to offer a protective effect.

Avaliação dos fatores de risco e proteção associados à elevação da pressão arterial em crianças / Evaluation of risk and protection factors associated with high blood pressure in children

FUNDAMENTO: Estudos epidemiológicos têm mostrado um aumento da prevalência da hipertensão arterial na faixa etária pediátrica. Hoje se sabe que os fatores de risco poderiam ter sido detectados na infância, o que auxiliaria na prevenção da doença. OBJETIVO: Avaliar fatores de risco e de proteção relacionados à elevação da pressão arterial na infância. MÉTODOS: Foram avaliadas crianças de 3 a 10 anos moradoras dos distritos sanitários leste e sudoeste de Goiânia, Goiás. Obtiveram-se os seguintes dados: peso ao nascer, aleitamento materno, história familiar de hipertensão e obesidade, peso, estatura, índice de massa corporal (IMC) e pressão arterial. Utilizaram-se os testes de U de Mann-Whitney para comparar a variação da pressão arterial quanto às variáveis descritas. RESULTADOS: Na amostra estudada, 519 crianças foram avaliadas, 246 (47,4 por cento) do sexo masculino. Avaliação do IMC identificou 109 (21 por cento) com excesso de peso, das quais 53 (10,3 por cento) eram obesas. O aleitamento materno predominante e/ou exclusivo por tempo inferior a 6 meses foi encontrado em 242 (51,2 por cento). As médias da pressão sistólica se encontraram significativamente mais elevadas naquelas crianças com aleitamento materno exclusivo e/ou predominante por tempo inferior a 6 meses (p = 0,04), história familiar positiva para hipertensão (p = 0,05) e excesso de peso (p < 0,0001). Esses dados foram confirmados na análise multivariada. CONCLUSÃO: Na amostra estudada, excesso de peso e fatores hereditários podem estar associados à elevação da pressão arterial, e o tempo em aleitamento materno superior a 6 meses parece conferir um efeito protetor.

BACKGROUND: Epidemiological studies have shown an increased prevalence of high blood pressure in pediatric patients. Today we know that risk factors can be detected during childhood and may help in preventing the disease. OBJECTIVE: To evaluate risk and protection factors related to high blood pressure in childhood. METHODS: We evaluated children aged 3 to10 years, residing in the east and southwest sanitary districts of the City of Goiânia, Goiás, and obtained the following data: birth weight, breastfeeding, family history of high blood pressure and obesity, weight, height, body mass index (BMI), and blood pressure. We applied the Mann-Whitney U-test to these variables in order to compare pressure variation. RESULTS: In this sample, 519 children were evaluated, and 246 (47.4 percent) of them were male. The BMI assessment identified 109 (21 percent) overweight children, of which 53 (10.3 percent) were obese. Predominant and/or exclusive breastfeeding for less than 6 months was found in 242 (51.2 percent). The mean systolic pressure was significantly higher in children with exclusive and/or predominant breastfeeding for less than 6 months (p = 0.04), and in children with family history of high blood pressure (p = 0.05), and in overweight children (p <0.0001). These data were confirmed in multivariate analysis. CONCLUSION: In this sample, we observed that overweight and hereditary factors may be associated with elevated blood pressure, and that breastfeeding for more than 6 months seems to offer a protective effect.

Anatomy of branches linguais of nerve hipoglosso in sus-scrofa domesticates, L., 1758 - DOI: 10.4025/actascibiolsci.v31i1.772 / Anatomia dos ramos linguais do nervo hipoglosso em Sus scrofa domesticus, L., 1758 - DOI: 10.4025/actascibiolsci.v31i1.772

We studied the ramifications and distribution of the hypoglossal nerve after it penetrated the root of the tongue (36 antimeres) of adult swines (Sus scrofa domestica, L. 1758) from both sexes slaughtered in frigorific (metropolitan region of Goiânia, Goiás - Brazil). The material was chilled for transportation (4C); placed in aqueous solution of formaldehyde (7%) for 72 hours; submerged in aqueous solution of nitric acid (15%) for 72 hours; dissected under magnifying glass (RASOR, II-20). The trunk and the primary, secondary and tertiary nervous branches were observed in the antimeres (method of Bittencourt et al., 1987). We followed the topography of the nerve starting from the posterior mandibular region between the myofascial structures of the mylohyoid and hypoglossal. On the root of the tongue the nerve crosses the lingual artery, becoming medial in relationship to the latter. It subdivides into three orders of dimension towards the superior longitudinal muscle as follows: left antimere, 4 to 13 primary branches, 4 to 21 secondary branches and 0 to 16 tertiary branches; on the right antimere, 8 to 18 primary branches, 3 to 13 secondary branches and 0 to 12 tertiary branches. In 27.59% of the analyzed antimeres, the branches of the hypoglossal nerve established joints with fibers from the lingual nerve of the correspondent antimere.

Estudamos as ramificações e distribuição do nervo hipoglosso após este penetrar na raiz da língua (36 antímeros) de suínos (Sus scrofa domesticus, L. 1758) adultos de ambos os sexos abatidos em frigorífico (região metropolitana de Goiânia, Estado de Goiás - Brasil). O material foi resfriado para transporte (4ºC), fixado em solução aquosa de formaldeído (7%) por 72h, imerso em solução aquosa de ácido nítrico (15%) por 72h, dissecado sob lupa (RASOR, II-20). Nos antímeros, observou-se o tronco e ramos nervosos primários, secundários e terciários (método de BITTENCOURT et al., 1987). Seguimos a topográfica do nervo, a partir da região retro mandibular, entre as estruturas miofaciais do milohioideo e hipoglosso. Na raiz da língua, o nervo cruza a artéria lingual, passando a ser medial em relação a esta. Subdivide-se em três ordens de grandeza em direção ao músculo longitudinal superior, como segue: antímero esquerdo, quatro a 13 ramos primários, quatro a 21 ramos secundários e zero a 16 ramos terciários; no antímero direito, oito a 18 ramos primários, três a 13 ramos secundários e zero a 12 ramos terciários. Em 27,59% dos antímeros analisados, os ramos do nervo hipoglosso estabeleceram junções com fibras do nervo lingual do antímero correspondente.

Anatomia dos ramos do nervo lingual em suíno / Anatomy of branches of nerve lingual in pigs

Neste trabalho, foram estudadas as ramificações e a distribuição do nervo lingual em 18 línguas de suínos adultos de ambos os sexos, sem especificação de raça. O material, colhido a fresco em rotina de abate, foi resfriado (4°C) para transporte, fixado em solução aquosa de formaldeído (7 por cento, 72 horas), imerso em solução aquosa de ácido nítrico (15 por cento, 72 horas), e dissecado sob lupa (RANSOR - II-20). Cada antímero dissecado possibilitou o registro dos ramos nervosos linguais primários, secundários e terciários presentes no interior das estruturas miofasciais do órgão, exibindo diferentes arranjos: o antímero esquerdo apresentou quatro a 10 ramos primários, quatro a 12 ramos secundários e zero a 11 ramos terciários. Já o antímero direito apresentou três a 8 ramos primários, zero a 11 ramos secundários e zero a 10 ramos terciários. Em 27,6 por cento das análises, os ramos linguais estabelecem associações com as fibras do nervo hipoglosso no antímero correspondente.

The distribution and ramifications of the lingual nerve of 18 tongues of adult pigs from both genders with no strain specification were studied. The material freshly collected in slaughter routine was cooled (4°C) for transportation, fixed in formaldehyde aqueous solution (7 percent, 72h), immerse in nitric acid aqueous solution (15 percent, 72h) and dissected with magnifying glass (RASOR, II-20). Each dissected antimere enabled the recording of primary, secondary and tertiary lingual nervous branches present inside the myofascial structure of the organ, exhibiting different arrangements: left antimere: 4 to 10 primary branches, 4 to 12 secondary antimeres and 0 to 11 tertiary branches; right antimere: 3 to 8 primary branches, 0 to 11 secondary antimeres and 0 to 10 tertiary branches. In 27.6 percent of analyses, the lingual branches established associations with the fibers of the hypoglossal nerve in the corresponding antimere.

O uso do corticóide altera o crescimento estatural na síndrome nefrótica primária? / Can steroid use change linear growth of children with primary nephrotic syndrome?

Objetivo: Avaliar, a médio prazo, o efeito do uso de corticóides sobre o crescimento linear de crianças portadoras de síndrome nefrótica primária (SNP).Métodos: Análise retrospectiva de crianças entre 2 e 8 anos portadoras de SNP em tratamento no serviço de Nefrologia Pediátrica do HC-FM-UFGo, que estavam em uso de prednisona por um período mínimo de um ano. Estudaram-se as variações anuais da velocidade do crescimento durante cinco anos de acompanhamento, individualizando-se os períodos com e sem uso de corticóide. O teste t-Student foi utilizado para avaliar as correlações das variáveis com o uso do corticóide. Resultados: Foram avaliadas 29 crianças, com média de idade de 63,4 + 36,4 meses. A velocidade de crescimento anual no período do uso de corticóide variou de 2,5 -18,1 cm, com média de 7,0+ 3,2, e na ausência do uso de corticóide variou de 1,31 - 13,9 cm, com média de 6,8 + 2,8. A comparação dos grupos pelo teste T-student não demonstrou diferença estatisticamente significante (p=0,81). Conclusão: O uso da prednisona em doses terapêuticas não demonstrou alterações na velocidade de crescimento em médio prazo nesse grupo de crianças.